Disseminated Low Grade Glioma in Children and Young Adults

Abstract

Low-grade gliomas [LGG] and in particular, pilocytic astrocytomas, are the most common of childhood tumours of the central nervous system [Bergthold et al 2014 [1], Gupta et al 2017 [2], Ryall et al 2017 [3]]. These are a mixed group of tumours with a World Heath Organization grading of I or II with a generally good outcome [Ryall, et.al, 2017 [3]. Leptomeningeal dissemination [spread via the cerebrospinal fluid [CSF] pathways] is rare with reported incidence of around 5% at diagnosis to 10% at progression [Chamidine et al 2016 [4], Dodgshun et al, 2016 [5], Yecies et al 2018 [6]] Hence, published data is limited on the incidence, natural history, patterns of dissemination or clinical outcome in both children and TYA with disseminated LGG [d-LGG]. While some reports suggest that children with d-LGG have an acceptable treatment outcome [Tsang et al 2017 [7], Chamidine et al 2016 [4], Bian et al 2013 [8], Perilongo et al 2003 [9], Hukin et al 2002], a few other published series depict a more dismal outlook [Von Hornstein et al 2011 [11], Rodriguez et al 2012 [12]]. As the natural history of the disease remains uncertain, it is unsurprising that there is also ambiguity with regard to the most effective treatment for children with d-LGG [Chamidine et al 2016 [4], Gnekov et al 2004 [13], Akar et al 2000 [14]]. In this report, we seek to initiate a dialogue on how this group of patients can best be managed using retrospectively gathered information on treatment outcomes in thirty-six children and adolescents with disseminated disease treated at the London Cancer Paediatric and Adolescent Neuro-Oncology Service [University College and Great Ormond street Hospitals, North London Cancer Network] UK.